@article{7584bf730fe04a2eb660fc45f42d6955,
title = "Systemic Epstein-Barr Virus-positive T-Cell Lymphoma of Childhood Presentation with Hemophagocytosis",
abstract = "A 2-year-old Asian girl presented to our facility for the evaluation of thrombocytopenia. She was treated with intravenous immunoglobulin under the impression of immune thrombocytopenia. However, her body temperature spiked and progressive pancytopenia, hepatosplenomegaly, abnormal liver function, coagulopathy, and pulmonary infiltration developed. The final diagnosis was systemic Epstein-Barr virus (EBV)-positive T-cell lymphoma of childhood with hemophagocytic syndrome. This type of cancer is extremely rare but occurs more commonly in Asians. Its prognosis is generally poor, and a treatment strategy is yet to be established. Double staining for EBV-encoded RNA and CD3 or CD8 is crucial for diagnosis. This type of lymphoma must be diagnosed differentially from acute EBV-associated hemophagocytic lymphohistiocytosis, which is considered nonmalignant. This case report highlights the importance of awareness of this type of rare cancer, a comprehensive diagnostic approach, and close communication between primary care physicians and pathologists.",
keywords = "Epstein-Barr virus, hemophagocytosis, T-cell lymphoma",
author = "Hung, {Giun Yi} and Yu, {Ting Yen} and Yen, {Hsiu Ju} and Yang, {Ching Fen} and Lin, {Li Yih} and Horng, {Jiun Lin}",
note = "Funding Information: Supported by a grant to Jiun-Lin Horng from the Ministry of Science and Technology, Taiwan (MOST 104-2311-B-038-002). The funder played no role in the study design, data collection and analysis, publication decision, or manuscript preparation. Funding Information: Received for publication August 26, 2018; accepted January 24, 2019. From the *Division of Pediatric Hematology and Oncology, Department of Pediatrics, Taipei Veterans General Hospital; †School of Medicine, National Yang-Ming University; ‡Department of Pathology and Laboratory Medicine, Taipei Veterans General Hospital; §Department of Life Science, National Taiwan Normal University; and ∥Department of Anatomy and Cell Biology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan. Supported by a grant to Jiun-Lin Horng from the Ministry of Science and Technology, Taiwan (MOST 104-2311-B-038-002). The funder played no role in the study design, data collection and analysis, publication decision, or manuscript preparation. The authors declare no conflict of interest. Reprints: Jiun-Lin Horng, PhD, Department of Anatomy and Cell Biology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, 250, Wuxing Street, Taipei 110, Taiwan, R.O.C. (e-mail: jlhorng@tmu.edu.tw). Copyright {\textcopyright} 2019 Wolters Kluwer Health, Inc. All rights reserved. Publisher Copyright: {\textcopyright} 2018 Wolters Kluwer Health, Inc. All rights reserved.",
year = "2019",
month = may,
day = "1",
doi = "10.1097/MPH.0000000000001452",
language = "English",
volume = "41",
pages = "319--320",
journal = "Journal of Pediatric Hematology/Oncology",
issn = "1077-4114",
publisher = "Lippincott Williams and Wilkins",
number = "4",
}
